{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:10:40Z","timestamp":1790745040774,"version":"4.1.0"},"reference-count":49,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T00:00:00Z","timestamp":1748563200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Russian Science Foundation","award":["23-64-00002"],"award-info":[{"award-number":["23-64-00002"]}]},{"name":"Russian Science Foundation","award":["# FGFG-2025-0017"],"award-info":[{"award-number":["# FGFG-2025-0017"]}]},{"name":"Ministry of Science and Higher Education of the Russian Federation","award":["23-64-00002"],"award-info":[{"award-number":["23-64-00002"]}]},{"name":"Ministry of Science and Higher Education of the Russian Federation","award":["# FGFG-2025-0017"],"award-info":[{"award-number":["# FGFG-2025-0017"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Phenylketonuria (PKU) is a common inherited metabolic disorder characterised by impaired metabolism of the amino acid phenylalanine. The disease results from a mutation in the phenylalanine hydroxylase (PAH) enzyme, which converts phenylalanine (Phe) into tyrosine (Tyr). The absence or inactivity of this enzyme results in significantly elevated levels of Phe in the blood, which can lead to severe neurological conditions, including intellectual disability, epilepsy, and other developmental disorders. Since its discovery, animal models have played a crucial role for understanding the pathophysiology of PKU, as well as providing recognisable proof of targets and surveying new remedial specialists and in vivo medicines. In the present study, we conducted a comprehensive review of the experimental and non-experimental animal models employed for phenylketonuria and its associated complications.<\/jats:p>","DOI":"10.3390\/ijms26115262","type":"journal-article","created":{"date-parts":[[2025,5,30]],"date-time":"2025-05-30T06:14:27Z","timestamp":1748585667000},"page":"5262","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Experimental Animal Models of Phenylketonuria: Pros and Cons"],"prefix":"10.3390","volume":"26","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7898-8977","authenticated-orcid":false,"given":"N. A.","family":"Bobrova","sequence":"first","affiliation":[{"name":"Federal Research Center for Innovator and Emerging Biomedical and Pharmaceutical Technologies, 125315 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. I.","family":"Lyubimova","sequence":"additional","affiliation":[{"name":"Moscow Center for Advanced Studies, Kulakova Str. 20, 123592 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D. M.","family":"Mishina","sequence":"additional","affiliation":[{"name":"Moscow Center for Advanced Studies, Kulakova Str. 20, 123592 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3764-2766","authenticated-orcid":false,"given":"V. S.","family":"Lobanova","sequence":"additional","affiliation":[{"name":"Morozov\u2019s Moscow City Children Clinical Hospital, Beregovoy Proezd 5A\/1, 121087 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. I.","family":"Valieva","sequence":"additional","affiliation":[{"name":"Morozov\u2019s Moscow City Children Clinical Hospital, Beregovoy Proezd 5A\/1, 121087 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9252-1601","authenticated-orcid":false,"given":"O. N.","family":"Mityaeva","sequence":"additional","affiliation":[{"name":"Federal Research Center for Innovator and Emerging Biomedical and Pharmaceutical Technologies, 125315 Moscow, Russia"},{"name":"Faculty of Fundamental Medicine, Moscow State University, Lomonosov Sky Pr., 27, 119991 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7608-439X","authenticated-orcid":false,"given":"S. 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